In eosinophilic granulomatosis with polyangiitis, granulomatous inflammation of the small blood vessels occurs. During this process, the tissues are infiltrated by eosinophilic granulocytes.
What is eosinophilic granulomatosis with polyangiitis?
In earlier times, eosinophilic granulomatosis with polyangiitis (EGPA) was also known as Churg-Strauss syndrome (CSS). The pathologists Jakob Churg (1910-2005) and Lotte Strauss (1913-1985) served as the namesakes. In the present day, however, the disease is known as eosinophilic granulomatosis with polyangiitis, or EGPA for short. Eosinophilic granulomatosis with polyangiitis (vasculitis) is a rare disease that belongs to the ANCA-associated vasculitides (AAV). ANCA is the abbreviation for anti-neutrophil cytoplasmic antibodies. ANCA-associated vasculitides are systemic diseases that can affect almost all organs. A typical feature of EGPA is involvement of the lungs, which is manifested by asthma symptoms. In eosinophilic granulomatosis with polyangiitis, granulomatous (granule-forming) inflammation of small and medium-sized blood vessels occurs. The tissue is infiltrated by eosinophilic granulocytes (inflammatory cells), which form a subset of white blood cells. Therefore, inflammation of the blood vessels occurs as an immune response. Women are twice as likely to develop EGPA as the male sex. In most cases, the disease manifests itself between the ages of 40 and 50. The incidence is one to two new cases per million population every year.
Causes
What causes eosinophilic granulomatosis with polyangiitis is still unknown. Various physicians suspect that it results from a malfunction of the body’s immune system. Allergic predispositions seem to play a special role. EGPA is particularly common in people who suffer from bronchial asthma or other allergies. In addition, the use of certain drugs such as montelukast is considered a risk factor for Churg-Strauss syndrome. A striking fact is that the damage to the blood vessels is caused by IgE antibodies. In addition, the disease always begins in the respiratory tract, suggesting an immune complex disease.
Symptoms, complaints, and signs
The symptoms of eosinophilic granulomatosis with polyangiitis occur in several phases. For example, at the onset of EGPA, chronic respiratory disease occurs. This may be asthma, allergic rhinitis, or sinusitis. While an arroded nasal septum is present in the nose, polyps occur in the sinuses. Later, bronchial asthma also occurs. In the second phase of EGPA, there is blood and tissue eosinophilia. The actual systemic disease becomes apparent only when vasculitis occurs in the small blood vessels. In most cases, it is accompanied by extravascular granulomas or hypereosinophilia. The factors that cause the different phases of the disease have not yet been clarified. Since eosinophilic granulomatosis with polyangiitis can occur in all organs, the gastrointestinal tract is sometimes affected. This is manifested by colicky symptoms, diarrhea, nausea and vomiting. Furthermore, inflammation of the coronary arteries or the heart muscle is possible, resulting in cardiac insufficiency or even a heart attack. It is not uncommon for individual nerve damage to appear, accompanied by stabbing pain, numbness and paralysis. If the skin is involved, bleeding the size of a pinhead, sores or the formation of ulcers occur. Other transient symptoms include fever, mild pneumonia, fatigue, and weight loss.
Diagnosis and course
The first suspicion of eosinophilic granulomatosis with polyangiitis arises in asthma patients when they suffer from other symptoms such as nerve damage or cardiac complaints. To ensure diagnosis, tissue samples are taken from the heart region, a nerve, or affected skin areas. Vasculitis can be identified by the typical composition of the inflammatory cells.A blood test reveals elevated levels of eosinophil granulocytes, which is a typical finding. A further indication is provided by an accumulation of IgE antibodies. These can be detected in about 40 percent of all patients. For the diagnosis of disease foci in the paranasal sinuses, lungs or heart that are not visible, imaging examination methods such as an X-ray examination, computer tomography (CT) or magnetic resonance imaging (MRI) are used. The life expectancy of EGPA patients has improved significantly in recent years with immunosuppressive treatments. However, relapses occur frequently, so that consistent control is necessary. With optimal treatment, the 5-year survival rate is more than 80 percent. However, death from myocardial infarction or heart failure occurs in some patients.
Complications
There is usually discomfort and complications of the respiratory tract due to the disease. The patient may equally suffer from inflammation of the nose and sinuses, which complicate everyday life and reduce the quality of life. Often, polyps also develop, which further complicate breathing. Due to the reduced oxygen supply, strenuous activities can no longer be performed and the affected person may lose consciousness. The heart and gastrointestinal tract are also affected, so that heart failure can occur. In the worst case, this leads to a heart attack and ultimately to death. The general feeling of illness also leads to fever and loss of appetite, which can lead to weight loss. In many cases, pneumonia also occurs. Treatment is usually causal with the help of antibiotics and leads to success after only a few days. However, early treatment is necessary to prevent secondary damage and other complications. There is no reduction in life expectancy. Complications can occur if there is a relapse and the patient contracts the disease again.
When should you see a doctor?
If breathing disorders occur, a medical examination should be initiated. If breathing stops, shortness of breath or interruptions in breathing occur, there is reason for concern. A visit to the doctor is necessary, as a life-threatening condition is imminent if the organism is persistently undersupplied with oxygen. Organ failure may occur, which is associated with irreparable and lifelong damage. If sleep disturbances occur with simultaneous shortness of breath, a doctor is needed as soon as possible. In case of high blood pressure, problems with the heart rhythm, palpitations, disturbances of concentration or attention deficits, a doctor should be consulted. Complaints of the sinus must be examined and treated as soon as they persist for several days or increase in intensity. If there are existing complaints of the stomach or intestines, a visit to the doctor is necessary. If nausea, vomiting or diarrhea symptoms occur repeatedly, a doctor should be consulted. In case of pain, cramps or paralysis all over the body, a doctor must be consulted as soon as possible. In severe cases, a comatose state is imminent if not treated. Numbness of the limbs is considered unusual and should also be medically clarified. If ulcers form, swelling occurs or a general feeling of illness, a visit to the doctor is necessary. If there is unwanted weight loss, persistent lassitude, or a feeling of physical weakness, a medical examination should be initiated.
Treatment and therapy
In the early stages of eosinophilic granulomatosis with polyangiitis, the patient is given antibiotics such as trimethoprim or sulfamethoxazole. In addition, low doses of anti-inflammatory corticosteroids are given, which bring about improvement in two-thirds of all patients. In advanced stages, patients also receive immunosuppressants in addition to corticosteroids. If the course of the disease is severe, corticosteroid shock treatment is considered promising. In this case, high doses of corticosteroids are administered in a few days in the form of an infusion. Immunoglobulins may also be used if this treatment is not successful. If the inflammation can be stopped by administration of the drugs, the cortisone dose is gradually reduced to the lowest possible administration. In this way, EGPA can be suppressed in the long term in most patients.
Outlook and prognosis
The prognosis for eosinophilic granulomatosis with polyangiitis-formerly known as Churg-Strauss syndrome-varies in dramatic severity. The more organs affected by the disease, the worse the prognosis. Problematically, this disease can strike virtually anyone, usually in midlife. The disease is described by inflamed blood vessels. Although only small and medium-sized vessels are affected, there are many of them. They lead to the organs and supply them with blood and nutrients. Insofar as vital organs such as the heart and brain, kidneys or nerves are affected by eosinophilic granulomatosis and polyangiitis, it becomes difficult. The prognosis is very poor without treatment. It often improves with medical treatment, but only with heavy medication. Corticosteroids or immunosuppressants such as azathioprine, methotrexate, or cyclophosphamide are often used. However, the prognosis in eosinophilic granulomatosis and polyangiitis is further worsened if these drugs have to be taken for a long time. Such preparations usually have severe side effects. These further weaken the already damaged organism. If the drugs can be slowly discontinued because the condition improves, the prognosis also improves. Unfortunately, the symptoms often recur. These can then turn out to be worse at the first onset of the disease.
Prevention
Preventive measures against eosinophilic granulomatosis with polyangiitis are not known. Thus, the causes of the disease have not yet been determined.
Follow-up
As a rule, no special or direct measures and options of aftercare are available to the affected person in this disease. In this regard, early diagnosis and detection of the disease is also important in the first place, so that other compilations can be avoided. The earlier the disease is detected, the better is the further course of the disease. It is not possible for the disease to heal on its own, so that the affected person is dependent on a doctor in any case. Only through proper medical treatment can the symptoms be permanently alleviated. The treatment of the disease is usually carried out with the help of medication. The affected person is dependent on the correct dosage and also on taking the medication regularly in order to alleviate the symptoms. A doctor should always be consulted if there is any uncertainty or if there are any questions. The doctor should also be contacted if undesirable side effects occur that have a negative impact on the quality of life of the person affected. In many cases, contact with other people affected by the disease can also be useful, as this often leads to an exchange of information. In most cases, the life expectancy of the affected person is not reduced by this disease.
This is what you can do yourself
In order not to burden the respiratory tract unnecessarily, the consumption of nicotine should be avoided. Likewise, do not visit premises where people smoke or where there are other pollutants in the air. To avoid further irritation of the gastrointestinal tract, toxins such as alcohol should not be consumed. In addition, care should be taken to maintain a healthy diet and avoid weight loss. Sufficient vitamins and fiber promote the recovery process and prevent malnutrition. The intake of carbohydrates, animal fats or olive oil should be avoided if possible, as they complicate the digestive process. The body must be supplied with sufficient fluids daily to prevent dehydration. Despite fatigue and exhaustion, it is important that the patient regularly spends time in the fresh air and gets enough exercise. To strengthen well-being, activities should be undertaken that have the effect of promoting a zest for life. Interaction with people from the immediate environment also helps to cope well with the challenges of everyday life. To reduce stress, it is advisable for the patient to use relaxation techniques such as yoga or meditation. The body needs an adequate supply of warmth. It is therefore advisable to avoid drafts or wearing clothing that is too thin.
